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Bile duct paucity in childhood—spectrum, profile, and outcome

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Abstract

We studied the etiological spectrum, clinicolaboratory and histological profile, and outcome of infants and children under 18 years of age presenting between December 2010 and May 2016 with histological evidence of paucity of intralobular bile ducts (PILBD, bile ducts to portal tract ratio < 0.6) Post-transplant PILBD was excluded. Of 632 pediatric liver biopsies screened, 70 had PILBD—44 were infants. PILBD was classified histologically into destructive (n = 50) and non-destructive PILBD (n = 20). Presentations were jaundice (98%), organomegaly (94%), pale stools (50%), and pruritus (43%). Infants had more cholestasis but less fibrosis on histology. Overall, 29 required liver transplantation (LT) for portal hypertension (n = 26), decompensation (n = 25), growth failure (n = 20), intractable pruritus (n = 5), and recurrent cholangitis (n = 2). Destructive PILBD has an odds for poor outcome (decompensation or need for LT within 1 year) of 1.53 (95% CI = 1.15–2.04). On binary logistic regression analysis, poor outcome was related to advanced fibrosis on liver biopsy [Exp (B) = 5.46, 95% CI = 1.56–19.04].

Conclusion: PILBD was present in 11% of pediatric liver biopsies and has a varied etiological spectrum. Destructive PILBD has poor outcome. Need for LT is guided by the presence of advanced fibrosis.

What is Known:

• Natural history of syndromic ductal paucity (Alagille syndrome) is complex.

• Duct loss is commonly seen with late presentation of biliary atresia.

What is New:

• The study classifies the etiological spectrum of ductal paucity histologically into destructive and non-destructive.

• Destructive duct loss carries poor prognosis regardless of the etiology of liver disease with subsequent need for liver transplantation.

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Abbreviations

ARC syndrome:

Arthrogryposis renal dysfunction cholestasis syndrome

AST:

Aspartate transaminase

CI:

Confidence interval

CPSS:

Congenital portosystemic shunt

GGT:

Gamma-glutamyl transpeptidase

INR:

International normalized ratio

IQR:

Interquartile range

PFIC:

Progressive familial intrahepatic cholestasis

PILBD:

Paucity of intralobular bile ducts

LB:

Liver biopsy

LT:

Liver transplantation

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Authors and Affiliations

  1. Department of Paediatric Hepatology, Institute of Liver and Biliary Sciences, D-1, Vasant Kunj, New Delhi, 110070, India

    Babu Lal Meena, Rajeev Khanna, Dinesh Rawat & Seema Alam

  2. Department of Pathology, Institute of Liver and Biliary Sciences, D-1, Vasant Kunj, New Delhi, 110070, India

    Chhagan Bihari & Archana Rastogi

Authors
  1. Babu Lal Meena
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  2. Rajeev Khanna
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  3. Chhagan Bihari
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  4. Archana Rastogi
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  5. Dinesh Rawat
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  6. Seema Alam
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Contributions

BLM collected data, BLM and RK analyzed data, CB and AR provided histopathological details, and BLM and RK prepared the first draft which was edited by RK and approved by all other authors. RK will be the guarantor of the manuscript.

Corresponding author

Correspondence to Rajeev Khanna.

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The authors declare that they have no conflict of interest.

Informed consent

Informed consent was obtained from all individual participants included in the study.

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Maintained.

Additional information

Communicated by Peter de Winter

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Meena, B.L., Khanna, R., Bihari, C. et al. Bile duct paucity in childhood—spectrum, profile, and outcome. Eur J Pediatr 177, 1261–1269 (2018). https://doi.org/10.1007/s00431-018-3181-3

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  • DOI: https://doi.org/10.1007/s00431-018-3181-3

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